Results 11 to 20 of about 22,551 (179)

Case Report: Diagnostic and Therapeutic Challenges in Severe Mechanobullous Epidermolysis Bullosa Acquisita

Frontiers in Immunology, 2022
Collagen VII is the main constituent of the anchoring fibrils, important adhesive structures that attach the epidermis to the dermal extracellular matrix.
Franziska Schauer, Alexander Nyström, Manfred Kunz, Stefanie Hübner, Sarah Scholl, Ioannis Athanasiou, Svenja Alter, Judith Fischer, Cristina Has, Dimitra Kiritsi   +9 more
doaj   +2 more sources

Epidermolysis bullosa acquisita: current diagnosis and therapy [PDF]

Dermatology Reports, 2011
Epidermolysis bullosa acquisita (EBA) is an acquired, autoimmune subepidermal blistering disease with an approximate prevalence of 0,2/million people. The hallmark of EBA is the presence of autoantibodies (mainly IgG class) to anchoring fibril collagen ...
Christine R. Mehren, Robert Gniadecki
doaj   +2 more sources

Mechanobullous form of epidermolysis bullosa acquisita: Insights into disease mechanisms as inferred by response to rituximab, but not to JAK inhibitors

JEADV Clinical Practice
Epidermolysis bullosa acquisita (EBA) is a rare autoimmune blistering disease associated with IgG autoantibodies directed against type VII collagen. Different clinical forms have been described, including the classical mechanobullous variant resembling ...
L. Gueissaz, A. Junge, R. Wolf, C. Schlapbach, L. Feldmeyer, L. Borradori   +5 more
doaj   +2 more sources

Childhood epidermolysis bullosa acquisita: Confirmation of diagnosis by skin deficient in Type VII Collagen, enzyme-linked immunosorbent assay, and immunoblotting

Indian Journal of Dermatology, 2016
Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disorder characterized by autoantibodies against Type VII collagen. It usually affects adults; childhood EBA is rare. We describe a 10-year-old girl presenting with recurrent tense
Nupur Goyal, Raghavendra Rao, C Balachandran, Sathish Pai, Balbir S Bhogal, Enno Schmidt, Detlef Zillikens   +6 more
doaj   +2 more sources

Epidermolysis Bullosa Acquisita in Children: Case Series [PDF]

Вопросы современной педиатрии, 2019
Background. Epidermolysis bullosa acquisita (EBA) is chronic disease accompanied with subepidermal blistering on skin and mucous membranes as a result of autoimmune aggression to type VII collagen.
Nikolay N. Murashkin, Leonid A. Opryatin, Alexander I. Materikin, Eduard T. Ambarchyan, Roman V. Epishev, Maria A. Nefedova   +5 more
doaj   +2 more sources

Epidermolysis Bullosa (EB) Acquisita in an Adult Patient with Previously Unrecognized Mild Dystrophic EB and Biallelic COL7A1 Mutations

Acta Dermato-Venereologica, 2018
Circulating anti-type VII collagen autoantibodies are frequently detected in patients with recessive dystrophic epidermolysis bullosa (RDEB). However, evidence supporting their pathogenic role in inducing epidermolysis bullosa acquisita (EBA) has been ...
Liliana Guerra, Angelo Giuseppe Condorelli, Paola Fortugno, Valentina Calabresi, Cristina Pedicelli, Giovanni Di Zenzo, Daniele Castiglia   +6 more
doaj   +2 more sources

Epidermolysis Bullosa Acquisita: The 2019 Update [PDF]

Frontiers in Medicine, 2019
Epidermolysis bullosa acquisita (EBA) is an orphan autoimmune disease. Patients with EBA suffer from chronic inflammation as well as blistering and scarring of the skin and mucous membranes.
Hiroshi Koga, Catherine Prost-Squarcioni, Hiroaki Iwata, Marcel F. Jonkman, Ralf J. Ludwig, Katja Bieber   +5 more
doaj   +2 more sources

Epidermolysis Bullosa Acquisita Occuring In A Patient With Systemic Lupus Erythematosus [PDF]

Saintika Medika, 2019
Epidermolysis Bullosa Acquisita (EBA) is a rare, chronic autoimmune subepidermal bullous disease and has been noted to be associated with systemic lupus erythematosus (SLE). The incidence of EBA and SLE in one patient within the period of 1980-1990 found
Lita setyowatie, Yustian Devika Rahmawati, Arif Widiatmoko, Diah Prabawati Retnani   +3 more
doaj   +2 more sources

European S2k guidelines on management of autoimmune blistering diseases in children and adolescents. [PDF]

J Eur Acad Dermatol Venereol
Autoimmune blistering disorders (AIBDs) in children are rare, challenging to diagnose and treat and often require immunosuppressants. Until now, no paediatric care guidelines existed. The EADV Task Force for AIBDs has developed the consensus‐based recommendations, enabling physicians to adopt a uniform, tailored treatment strategy to improve outcomes ...
Nanda A, Tedbirt B, Bodemer C, Bolling MC, El Hachem M, Nikolić M, Benton E, Caproni M, De D, Didona D, Drenovska K, Goebeler M, Hertl M, Horváth B, Kasperkiewicz M, Kiritsi D, Larralde M, Marzano AV, Meijer J, Murrell DF, Powell J, Sprecher E, Chandran NS, Tomayko MM, Uzun S, van Beek N, Ott H, Patsatsi A, Torrelo A, Welfringer-Morin A, Schmidt E, Joly P.   +31 more
europepmc   +2 more sources

Epidermolysis Bullosa Acquisita [PDF]

, 2022
Epidermolysis bullosa acquisita (EBA) is a subtype of pemphigoid that may present with scarring similar to hereditary dystrophic epidermolysis bullosa, of which the naming of EBA was derived.
Marcel F. Jonkman, Jonkman, Marcel F., Joost M. Meijer, Meijer, Joost M.; id_orcid   +3 more
core   +1 more source